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Dr. Mashfiqul Hasan
      Resident, MD Phase A (EM)
Discipline of Endocrine Medicine
                         BSMMU
   Case summary
   Short discussion
   7 year
   Boy
   Only child of parents
   Appearance of pubic hair, facial hair
   Gradual enlargement of phallus
   Deepening of the voice

          For 5-6 months
   No history of headache, visual disturbance or
    seizure.
   No significant past illness, no regular medication.
   No history of early onset puberty in family.
   Pubic hair :
    Slightly
    curled, dark,
    coarse,
    spread
    sparsely.
   Tanner stage
    of pubic
    hair: P3
   Testis: 15 ml on
    both sides, firm,
    symmetrical,
    smooth surface
   Stretched penile
    length: 12.5 cm
   Tanner stage of
    genitalia: G4
Height:
143 cm
   Current height :     143 cm
   Father’s height :    158 cm
   Mother’s height :    151 cm
   Expected adult height:



   So, the expected adult height is :
    ◦ ` 161 cm ( 10cm)
   Accelerated (>1 year)
   S. Testosterone          4.9 nmol/L
                      (0.1-1.0 nmol/l for 6-9 years)
   S. LH                    2.27 IU/L
                      (0.01-0.78 nmol/l for 8-10 years)
   S. FSH                   3.26 IU/L
                      (0.2–1.67 IU/L for 8-9 years)
   LH spike (>10 mIU/ml) after 30 minutes.
   No significant abnormality.
   Central idiopathic precocious
    puberty
   Inj. Decapeptyl (11.25 mg) 3 monthly
   Plan is to continue up to 11 years of age
   Now he is on regular follow up
   Pulsatile secretion of gonadotropin-releasing
    hormone (GnRH) and activation of the
    hypothalamo–pituitary–gonadal axis

   Lower end of the normal range for the onset of
    puberty:
    ◦ 8 years in girls and
    ◦ 9 years 6 months in boys
Central or
Gonadotropin dependent


     Peripheral or
Gonadotropin independent
   Short adult stature due to early epiphyseal
    fusion,
   Underlying pathology
   Adverse psychosocial outcomes
 Potential
          for    Evaluation
                          of
 progression     mechanism
   50% of cases regress or stop progressing, and
    no treatment is necessary
   Evaluation is needed when
    ◦ Progression through pubertal stages
    ◦ Growth velocity
    ◦ Bone age
    ◦ LH peak after GnRH agonist
   Clinical
   Lab investigations
    ◦
   Family history
   Features of CNS lesion
   Testicular size
   Features of specific cause
   S. Testosterone/S. Estradiol
   S. LH, S. FSH
   GnRH stimulation test
   S. ß-hCG
   S. DHEAS
   S. 17-hydroxy Progesterone
   Thyroid function test
   Pelvic ultrasound
   Testicular ultrasound
   MRI of brain
   GnRH agonists
    ◦ Triptorelin (Decapeptyl)


   Management of CNS lesion
   Removal of the cause
   Social stigmata, psychosocial
    impact
   Clinical dilemma
   Rational approach
THANK YOU

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A young boy with signs of puberty

  • 1. Dr. Mashfiqul Hasan Resident, MD Phase A (EM) Discipline of Endocrine Medicine BSMMU
  • 2. Case summary  Short discussion
  • 3.
  • 4. 7 year  Boy  Only child of parents
  • 5. Appearance of pubic hair, facial hair  Gradual enlargement of phallus  Deepening of the voice  For 5-6 months
  • 6. No history of headache, visual disturbance or seizure.  No significant past illness, no regular medication.  No history of early onset puberty in family.
  • 7. Pubic hair : Slightly curled, dark, coarse, spread sparsely.  Tanner stage of pubic hair: P3
  • 8. Testis: 15 ml on both sides, firm, symmetrical, smooth surface  Stretched penile length: 12.5 cm  Tanner stage of genitalia: G4
  • 10. Current height : 143 cm  Father’s height : 158 cm  Mother’s height : 151 cm  Expected adult height:  So, the expected adult height is : ◦ ` 161 cm ( 10cm)
  • 11.
  • 12. Accelerated (>1 year)
  • 13. S. Testosterone 4.9 nmol/L (0.1-1.0 nmol/l for 6-9 years)  S. LH 2.27 IU/L (0.01-0.78 nmol/l for 8-10 years)  S. FSH 3.26 IU/L (0.2–1.67 IU/L for 8-9 years)
  • 14. LH spike (>10 mIU/ml) after 30 minutes.
  • 15. No significant abnormality.
  • 16. Central idiopathic precocious puberty
  • 17. Inj. Decapeptyl (11.25 mg) 3 monthly  Plan is to continue up to 11 years of age  Now he is on regular follow up
  • 18.
  • 19. Pulsatile secretion of gonadotropin-releasing hormone (GnRH) and activation of the hypothalamo–pituitary–gonadal axis  Lower end of the normal range for the onset of puberty: ◦ 8 years in girls and ◦ 9 years 6 months in boys
  • 20. Central or Gonadotropin dependent Peripheral or Gonadotropin independent
  • 21. Short adult stature due to early epiphyseal fusion,  Underlying pathology  Adverse psychosocial outcomes
  • 22.  Potential for  Evaluation of progression mechanism
  • 23. 50% of cases regress or stop progressing, and no treatment is necessary  Evaluation is needed when ◦ Progression through pubertal stages ◦ Growth velocity ◦ Bone age ◦ LH peak after GnRH agonist
  • 24. Clinical  Lab investigations ◦
  • 25. Family history  Features of CNS lesion  Testicular size  Features of specific cause
  • 26. S. Testosterone/S. Estradiol  S. LH, S. FSH  GnRH stimulation test  S. ß-hCG  S. DHEAS  S. 17-hydroxy Progesterone  Thyroid function test
  • 27. Pelvic ultrasound  Testicular ultrasound  MRI of brain
  • 28.
  • 29. GnRH agonists ◦ Triptorelin (Decapeptyl)  Management of CNS lesion
  • 30. Removal of the cause
  • 31. Social stigmata, psychosocial impact  Clinical dilemma  Rational approach
  • 32.

Notas do Editor

  1. results when pulsatile secretion of gonadotropin-releasing hormone (GnRH) is initiated and the hypothalamo–pituitary–gonadal axis is activated.
  2. No CNS lesion: IdiopathicCNS lesion: hypothalamic hamartoma, other hypothalamic tumor, malformations, injury
  3. Progression from one stage to the next in 3–6 month.Accelerated (> about 6 cm per yr)Usually advanced by at least 1 yr
  4. Testicular volume. Signs of specific cause.
  5. 4-wk and 12-wk preparations (intramuscular); 3.00–3.75 mg every 4 wk (1-mo depot) or 11.25 mg every 12 wk (3-mo depot)